Spinal muscular atrophy type II (intermediary) and III (Kugelberg-Welander): evolution of 50 patients with physiotherapy and hydrotherapy in a swimming pool

Abstract

-We added hydrotherapy to 50 patients with spinal muscular atrophy (SMA) who were being treated with individual conventional physiotherapy. Hydrotherapy was performed at an approximate temperature of 30 degrees Celsius, twice a week, for thirty minutes in children and for forty-five minutes in adults during a 2-year period. The outcome derived from this combined modality of treatment was rated according to physiotherapeutic evaluations, the MMT (Manual Muscular Test), and the Barthel Ladder. Patients were reevaluated at 2-month intervals. After two years of ongoing treatment, we were able to observe that the deformities in hip, knee and foot were progressive in all SMA Type II patients, and in some Type III. Muscle strength stabilized in most SMA Type III patients, and improved in some. MMT was not done in SMA Type II. In all patients we were able to detect an improvement in the Barthel Ladder scale. This study suggests that a measurable improvement in the quality of daily living may be obtained in patients with SMA Types II and III subjected to conventional physiotherapy when associated with hydrotherapy.KEY WORDS: physiotherapy, hydrotherapy, spinal muscular atrophy.Spinal muscular atrophy (SMA) manifests as diffuse muscle weakness secondary to degeneration of the anterior horn cells and bulbar nuclei without pyramidal tract involvement 5,6,7 . Atrofia muscular espinhal tipo II (intermediária) e III (Kugelberg-Welander): evolução de 50 pacientes com fisioterapia e hidroterapia em piscinaRESUMO -A hidroterapia foi realizada em SO pacientes com atrofia muscular espinhal, os quais foram também tratados com fisioterapia individual convencional. O tratamento hidroterápico foi realizado em piscina aquecida numa temperatura de aproximadamente 30° Celsius, duas vezes por semana, durante 30 minutos em crianças e 45 minutos em adultos num período de dois anos. Os benefícios deste tipo de tratamento foram avaliados de acordo com a evolução clínica, o MMT(Teste de Força Muscular) e a Escala de Barthel. Os pacientes foram reavaliados a cada dois meses. Após dois anos de tratamento nós observamos que as deformidades nos quadris, joelhos e pés foram progressivas em todos os pacientes do Tipo II e em alguns do Tipo III. Houve estabilização da força muscular na maioria dos pacientes com SMA Tipo III, e melhora da força em alguns; nos pacientes com SMA Tipo II este teste não pode ser realizado. Em todos os pacientes pudemos verificar melhora em realção à Escala de Barthel. Este estudo sugere que houve melhora destes pacientes com SMA Tipos II e III em relação as atividades de vida diária quando a fisioterapia convencional foi associada a hidroterapia. PALAVRAS-CHAVES: fisioterapia, hidroterapia, atrofia muscular espinhal.

Exercise in neuromuscular disease

“…In a study of 50 patients with SMA 2 and 3 followed for 2 years, muscle strength stabilized in all SMA 2 patients and in some SMA 3 patients. All reported improvement on the Barthel Ladder scale, despite progression of existing joint deformities in a majority of patients . A case report described a 3‐year‐old girl with SMA 3 who received aquatic therapy twice‐weekly for 14 weeks, with a corresponding 11% improvement on the Gross Motor Function Measure, along with faster velocity and increased stride length on gait testing …”

“…All reported improvement on the Barthel Ladder scale, despite progression of existing joint deformities in a majority of patients. 52 A case report described a 3-year-old girl with SMA 3 who received aquatic therapy twiceweekly for 14 weeks, with a corresponding 11% improvement on the Gross Motor Function Measure, along with faster velocity and increased stride length on gait testing. 53 A minority of patients who survive Poliomyelitis develop sequelae decades after their initial illness, with new weakness, fatigue, and pain.…”

Physical exercise training for type 3 spinal muscular atrophy

“…The target frequency was fully achieved in one study (Vry 2014), partly achieved in two studies (Lewelt 2015;Madsen 2015), and not reported in six studies (Basoglu 2006;Cunha 1996;Dahl 2004;McCartney 1988;Milner-Brown 1988;Salem 2010). Descriptions of training parameters were incomplete: in four studies with regards to intensity (Basoglu 2006;Cunha 1996;Dahl 2004;Salem 2010), and in three studies with regards to time (Basoglu 2006;McCartney 1988;Milner-Brown 1988). Four studies reported on the occurrence of adverse events (Lewelt 2015;Madsen 2015;McCartney 1988;Vry 2014).…”

“…We excluded nine studies that were not RCTs (see Characteristics of excluded studies). The interventions were strength training alone in four studies (Basoglu 2006;Lewelt 2015;McCartney 1988;Milner-Brown 1988), aerobic exercise training alone in one study (Madsen 2015), aquatic therapy incorporated in a comprehensive rehabilitation program in three studies (Cunha 1996;Dahl 2004;Salem 2010), and a combination of functional strengthening exercises and whole body vibration in one study (Vry 2014). Strength training consisted of concentric resistance training either alone (in McCartney 1988 andMilner-Brown 1988) or in combination with gravity-eliminated movements (in Basoglu 2006 andLewelt 2015).…”

“…Four studies included participants with other neuromuscular diseases, such as muscular dystrophies, polyneuropathies, and myopathies (Dahl 2004;McCartney 1988;Milner-Brown 1988;Vry 2014). Four studies used home-based exercise programs (Basoglu 2006;Lewelt 2015;Madsen 2015;Vry 2014), in four studies participants exercised at an outpatient clinic or university (Cunha 1996;Dahl 2004;McCartney 1988;Salem 2010), and one study did not specify the setting (Milner-Brown 1988). Most studies were limited by inadequate research design: one was a non-randomized controlled clinical trial (Madsen 2015), seven were multiple-case studies (Basoglu 2006;Cunha 1996;Dahl 2004;Lewelt 2015;McCartney 1988;Milner-Brown 1988;Vry 2014), and one was a single-case study (Salem 2010).…”